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PACES 2024 > Station 2/5: Rheumatology > Flashcards

Station 2/5: Rheumatology Flashcards

1
Q

what are the 1997 Revised ACR diagnostic criteria for SLE?

A

need 4 out of 11

DOMP CRASH AI

Cutaneous features:
1. Malar rash (sparing nasolabial folds)
2. Discoid rash (scarring)
3. Photosensitivity
4. Oral Ulcers

Systemic
5. Arthritis: non erosive (>/= 2 peripheral joints)
6. serositis: pleuritis, pericarditis, peritonitis
7. Renal involvement: proteinuria, cellular cast, renal impairment
8. CNS: psychosis, seizures

Laboratory
9. Haematological cytopenias: leukopenia, lymphopenia; haemolytic anaemia; thrombocytopenia
10. Anti-Nuclear antibody positive
11. Immunological:
- antiphospholipid antibodies (anticardiolipin antibody, lupus anticoagulant, false + VDRL)
- anti dsDNA, anti-Smith

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2
Q

what are the cutaneous features that are part of the diagnostic criteria for SLE?

A
  1. Malar rash (sparing nasolabial folds)
  2. Discoid rash (scarring)
  3. Photosensitivity
  4. Oral Ulcers
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3
Q

what are the systemic features that are part of the diagnostic criteria for SLE?

A
  1. Arthritis: non erosive (>/= 2 peripheral joints)
  2. serositis: pleuritis, pericarditis, peritonitis
  3. Renal involvement: proteinuria, cellular cast, renal impairment
  4. CNS: psychosis, seizures
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4
Q

what are the laboratory features that are part of the diagnostic criteria for SLE?

A

Laboratory
9. Haematological cytopenias: leukopenia, lymphopenia; haemolytic anaemia; thrombocytopenia
10. Anti-Nuclear antibody positive
11. Immunological:
- antiphospholipid antibodies (anticardiolipin antibody, lupus anticoagulant, false + VDRL)
- anti dsDNA, anti-Smith

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5
Q

what are some other features of SLE not classically in the diagnostic criteria?

A

fever
LOW
alopecia
livedo reticularis
vasculitis
Raynauds phenomenon

sjogrens
anti-phospholipid syndrome
interstitial lung disease

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6
Q

what other conditions are commonly associated with SLE?

A

Raynaud’s phenomenon
Sjogren’s
Anti-phospholipid syndrome
Interstitial Lung disease

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7
Q

what is an overlap syndrome?

A

presence of clinical features and autoantibodies of >/= connective tissue disorders

includes mixed connective tissue disease and scleromyositis - exact diagnosis depends on which disease patient shows predominant symptoms

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8
Q

history taking in SLE: what to ask for?

A
  • rash: distribution, photosensitivity, livedo reticularis
  • alopecia
  • oral, genital ulcers
  • sicca symptoms: dry eyes/ mouth
  • joint pain/ swelling: distribution/ deformity
  • early morning stiffness: duration, improvement with exercise
  • digitial gangrene (vasculitis)
  • serositis: chest / abdo pain
  • fatiguability, lethargy
  • hx of renal impairment, proteinuria (frothy urine, LL swelling)
  • hx CNS disorder- seizures, psychosis
  • hx of anaemia
  • blood clots/prothrombotic: TTP, APS, PE, DVT

AI screen:
fever, LOW, LOA
Raynaud’s phenomenon
Telangiectasia
SOB from ILD

Treatment complications

PMH, Drug hx, FHx, Social Hx, obstetric hx

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9
Q

what other PMH etc to ask for in history taking for SLE?

A

PMH
med hx, drug allergy and TCM use
smoking, ETOH
family hx of thyroid/ autoimmune disorder
job, hobbies
functional impairment and impact on ADL
plans for pregnancy

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10
Q

what to ask for re: treatment complications in patient with SLE?

A

cushingoid, hirsutism, cataracts
hx of DM, HTN, HLD
hx of osteoporosis, AVN fem head, mood disturbances
anaemia- SOBOE, postural giddiness, GI bleed, lethargy
Hx CKD, transaminitis
Maculopathy 2’ HCQ

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11
Q

what to examine for in patient with SLE?

A

General appearance

Hands

Upper limbs

Face

Cardio-Respi

Rest of body

Function

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12
Q

what to examine for in patient with SLE?
(General appearance)

A

cushingoid, xanthelasma, DM dermopathy
Facial malar rash (sparing nasolabial folds)
alopecia (beware of wig!)
look for possible overlap syndrome/ MCTD (features of RA, SSc, Myositis)

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13
Q

what to examine for in patient with SLE?
(Hands)

A

Arthritis/ arthralgia
Jaccoud’s arthropathy
Livedo reticularis
Vasculitic changes, digitial gangrene, splinter haemorrhages, nail fold capillaries
nail changes, periungual erythema/ infarcts
Raynauds
Scars from discoid rash, active discoid lupus including over palm
palmar erythema

overlap: sclerodactyly, RA hands
double pinch test for thin skin (steroids)

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14
Q

what to examine for in patient with SLE?
upper limbs

A

rash, discoid scarring
proximal myopathy (steroids)

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15
Q

what to examine for in patient with SLE?
(face)

A

malar rash
pinched facies?-> microstomia? (SSc overlap)

hairline: alopecia (may be scarring from discoid rash)
salt and pepper pigmentation

ears for follicular plugging

eyes:
- scleritis, episcleritis
- conjunctiva for pallor, sclera icterus (haemolytic anaemia)
- cataracts (steroids)

mouth: oral ulcers, thrush from steroids

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16
Q

what to examine for in patient with SLE?
(cardio-respi)

A

cardio: pericardial rub, libbman sack’s endocarditis, pulmonary hypertension

respi: ILD, pleural effusion, pleural rub

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17
Q

what to examine for in patient with SLE?
rest of body

A

rash
petechiae (thrombocytopenia)
striae (steroids)
lower limbs for pitting oedema (hypoalbuminuria, proteinuria)

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18
Q

what to examine for in patient with SLE?
assessing function

A

assess hand function if sclerodactyly and deformities present

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19
Q

SLE examination of patient; how to complete your examination?

A

complete examination by:
- doing complete physical examination of all joints, abdomen for organomegaly
- urinalysis for proteinuria, haematuria and cellular casts
- take BP
- asking for history of color change in hands in the cold (Raynauds)

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20
Q

features of mixed connective tissue disease?

A

combines features of SLE, RA, systemic sclerosis, myositis

clinical features include:
- raynauds’
- sclerodactyly
- joint pain/ swelling
- rash
- malaise
- sjogrens
- myositis
- pulmonary hypertension
- pleuritis, pericarditis
- oesophageal hypomotility
- leucopenia

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21
Q

what antibodies are associated with mixed connective tissue disease?

A

anti-RNP antibody and speckled pattern ANA

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22
Q

prognosis of mixed connective tissue disease?

A

better prognosis
less involvement of internal organs
may progress to predominance of one connective tissue disorder over time

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23
Q

ix of SLE?

A

inx to confirm diagnosis, determine severity of organ involvement and activity of disease

Diagnosis:
FBC: leukopenia, anaemia (could be haemolytic), thrombocytopenia
Renal panel, Urinalysis: renal impairment, proteinuria, microscopic haematuria, cellular casts
ANA
Anti-dsDNA, Anti-Sm, Anti lymphocyte antibodies, anti centromere antibodies

Severity:
C3, C4
ESR
Albumin

Organ involvement:
Renal US, biopsy
CXR, HRCT
ECG, 2DE

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24
Q

when to consider renal biopsy in SLE?

A

if renal impairment present +/-
proteinuria, microscopic haematuria, cellular casts

may need renal biopsy to evaluate class of lupus nephritis
-> Class III, IV and V require treatment with high dose steroids/ rituximab. Class VI usually not treatable

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25
Q

What is the WHO classification of lupus nephritis?

A

Class I: Minimal change disease
Class II: Mesangial
Class III: Focal proliferative
Class IV: Diffuse proliferative
Class V: Membranous
Class VI: Glomerulosclerosis/ advance diffuse sclerosing

Minced Meat For De Meat Grinder

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26
Q

Serological tests to send for SLE?

A

anti nuclear antibody: sensitive screening test
-> will get some false positives

anti-dsDNA: specific
anti-smith: specific

anti-phospholipid antibodies: helpful in diagnosis and to evaluate for associated anti-phospholipid syndrome
- lupus anticoagulatn
- anti-cardiolipin
- anti-B2 glycoprotein

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27
Q

what blood tests to send to evaluate for assesment of activity of SLE

A

low complements: C3, 4
raised ESR
CRP: usually normal or mildly elevated in SLE, unless in presence of concurrent infection
low albumin

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28
Q

renal ix in SLE?

A

UFEME, urine protein:Cr, 24h urine total protein

renal US
renal biopsy

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29
Q

respiratory inx in SLE?

A

CXR, HRCT -> to look for interstitial lung disease, pleural effusions (serositis)

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30
Q

Cardiac ix in SLE

A

troponins
ECG
2DE

-> pericarditis, libbman sacks endocarditis, pulmonary hypertension, pericardial effusion

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31
Q

management of SLE?

A

MDT approach

patient education: avoid sunlight, support group

pharmacological treatment:
definitive immunosuppression: corticosteroids, steroid sparing agents, anti-CD20 monoclonal antibodies ie. Rituximab

cutaneous manfestations:
avoid sunlight, use sunscreen
hydroxychloroquine

symptomatic meds: NSAIDs for arthritis (but avoid in lupus nephritis)

anticoagulation for anti-phospholipid syndrome

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32
Q

immunosuppression agents used for SLE?

A

corticosteroids:
high dose IV methylprednisolone for rapidly progressing lupus nephritis or very active lupus e.g. Diffuse alveolar haemorrhage, gut vasculitis

steroid sparing agents:
cyclophosphamide
mycophenolate mofetil

Rituximab (anti-CD20) - indicated for rapidly progressive lupus nephritis

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33
Q

what medications may cause drug-induced lupus?

A

procainamide, hydralazine, isoniazid

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34
Q

features of drug-induced lupus

A

usually multi-organ involvement, but usually spares CNS and renal

clinical and lab features usually return to normal after withdrawal of offending drug

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35
Q

what antibody is associated with drug-induced lupus?

A

anti-histone antibody
- but not specific for it

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36
Q

what is Jaccoud’s arthropathy?

A

chronic, reducible, non-erosive, painless joint deformities with preservation of hand function

characterized by ulnar deviation of 2-5th fingers and subluxation of the MCPJ, which are voluntarily correctable by the patients

toes may also be affected

deformities corrected by placing hands flat on table

caused by imbalance between flexor and extensor tendons

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37
Q

causes of Jaccoud’s arthropathy?

A

initially described as complication of recurrent rheumatic fever

connective tissue disease, SLE, psoriatic arthritis
inflammatory bowel disease
malignancy
familial mediterranean fever

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38
Q

what are the types of scleroderma?

A

systemic sclerosis
1) Limited cutaneous SSc
- includes CREST syndrome (calcinosis, Raynaud’s, oesophageal dysmotility, sclerodactyly, telangiectasia) that has a better prognosis

2) Diffuse cutaneous SSc
- independent of whether there is systemic involvement, only considers skin
- face and neck not considered

3) systemic sclerosis sine scleroderma
- systemic complcications without skin involvement

localised scleroderma
- morphea
- linear scleroderma

overlap syndromes

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39
Q

differences between diffuse cutaneous and limited cutaneous systemic sclerosis?

A

skin involvement:
distal and proximal aspects of extremities, trunk +/- face (diffuse)
vs
distal to elbows and knees but may involve face and neck (limited)

Raynaud’s phenomenon:
onset within 1 year or at time of skin changes (diffuse)
vs
may precede skin disease by years (limited)

Organ involvement:
renovascular hypertensive crisis, interstitial fibrosis, GI, cardiac (diffuse)
vs
GI, pulmonary arterial hypertension after 10-15 years of disease in < 10% of patients, biliar cirrhosis (limited)

Nail fold capillaries:
dilation and dropout (diffuse) vs dilation without significant dropout (limited)

Antinuclear antibodies:
Anti-topoisomerase 1 antibodies (Scl-70) - diffuse, assoc w pul fibrosis
vs
Anti-centromere Ab (ACA) - limited

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40
Q

what antibodies are associated with diffuse cutaneous vs limited cutaneous systemic sclerosis

A

Anti-topoisomerase 1 antibodies (Scl-70) - diffuse, assoc w pul fibrosis
vs
Anti-centromere Ab (ACA) - limited

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41
Q

types of localized scleroderma

A

morphea - plaque of indurated mauve skin which later becomes waxy and either pale or hyperpigmented with absent hair and sweating

linear scleroderma

en coup de sabre- linear scleroderma that presents on the frontal or frontoparietal scalp

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42
Q

treatment for morphea?

A

may improve spontaneously

topical steroids, topical/ oral vit D analogues and phototherapy may be helpful

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43
Q

history taking for scleroderma?

A

skin:
- skin tightness: duration, extent, progression
- tight facial skin
- sicca symptoms
- vitiligo, salt & pepper pigmentation
- fingers: tighntess, limited ROM, digitial ulceration and infarcts/ gangrene
- nail changes: atrophic and breaking of nails
- calcinosis

Vascular:
- raynauds phenomenon: triggers- smoking, cold, stress
- telangiectasia

MSK:
- joint pain: distribution, deformity
- weakness (myositis)

GI:
- reflux symptoms, dysphagia, +/- NGT and LOW +/- hoarse voice
- malabsorption, steatorrhoea

cardio/resp/renal
- SOB, chest pain, dry cough (CMP, pHTN, ILD, anaemia)
- hx of renal crisis: stroke, eye, renal sequelae

AI screening

Treatment and complications

PMH, drug hx, family hx etc

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44
Q

history elements to screen for in scleroderma to suggest other autoimmune conditions

A

early morning stiffness- duration, improvement with exercise

rashes- location, photosensitivity

alopecia

oral/ genital ulcers

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45
Q

history for scleroderma: treatment and complications

A

history of IV prostaglandin, nitroprusside
digital gangrene, infarct, amputations
steroid therapy and cushings, hirsutism, cataracts
hx of DM, HTN, HLD

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46
Q

history in scleroderma: what other PMH/FHx/Social hx to ask for

A

past medical history
meds: drug allergy, TCM use
smoking, ETOH use
Family history: thyroid, autoimmune illness (SLE, RA)
Job, hobbies
- functional impairment and impact on ADLs

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47
Q

Examination findings in scleroderma: general appearance

A
  • pinched avian facies
  • tight shiny skin
  • thin/cachectic (malabsorption)
  • NGT (dysphagia)
  • amputations or gangrene of toes (Raynauds/ vasculitis) or surgical amputations for superimposed wet gangrene
  • respiratory distress, supplemental O2 (pulmonary fibrosis, pulmonary hypertension)
  • salt and pepper pigmentation, vitiligo
  • IV infusion wrapped in aluminium/ black cloth (prostaglandin or nitroprusside)
  • if cushingoid -> think ILD or overlap syndrome! look for complications of steroid therapy
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48
Q

examination findings in scleroderma: hands

A
  • calcinosis
  • Raynaud’s
  • infarcts of digits: digital pulp atrophy, digital pitting, nail bed infarcts
  • sclerodactyly/ tight shiny skin (with tapering of fingers)
  • telangiectasia: esp over nail folds of fourth digit
  • nail changes: breaking of nails, atrophic nails
  • wasting of intrinsic hand muscles (disuse)

palpate for:
tight skin with double pinch test (check dorsum of hands, forearm, arm, trunk, forehead)

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49
Q

examination findings in scleroderma: arms

A

tight skin (extent of involvement)
proximal myopathy (myositis)

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50
Q

examination findings in scleroderma: face

A
  • telangiectasia
  • conjunctival palllor (anaemia)
  • eye closure: difficulty closing eyes
  • perioral puckering
  • microstomia
  • dry mouth and poor dentition
  • oral thrush
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51
Q

examination findings in scleroderma: chest

A

pulmonary fibrosis: velcro like end inspiratory crepitations bibasally

auscultate the heart: pulmonary hypertenison, dilated cardiomyopathy, pericardial effusion, AV blocks

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52
Q

examination findings in scleroderma: lower limbs

A
  • calcinosis, raynaud’s, ulcerations, telangiectasia
  • gangrene, amputations, digitial infarcts
  • if above present, check pulses
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53
Q

examination findings in scleroderma: function of hands

A
  • turn door knob/ open bottle (Grip function)
  • pick up coin (pincer grip)
  • button clothes
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54
Q

examination in scleroderma: how to complete examination

A
  • BP for hypertension
  • auscultate abdomen for renal bruit (RAS)
  • fundoscopy for hypertensive retinopathy and papilloedema
  • urine dipstick for proteinuria and haematuria
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55
Q

ix for scleroderma

A

Diagnostic:
- antiobodies: anti-centromere (limited cutaneous), anti-topoisomerase (diffuse)
- nail fold capillary microscopy: fewer capillaries than normal, numerous dilated capillary loops

assess severity and complications:
- ESR, CRP elevated
- CK: elevated in myositis
- XR hands: acral osteolysis, calcinosis
- ECG, 2DE: AV block, dilated CMP, heart failure
- CXR/ HRCT: pulmonary fibrosis
- pulmonary function tests
- OGD: strictures, reflux

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56
Q

antibodies associated with scleroderma?

A
  • anti-centromere antibodies: 45-50% of patients with limited cutaneous SSc, rare in diffuse
  • anti-DNA topoisomerase I antibodies (Scl-70): 30% of diffuse, assoc pulmonary fibrosis. when present in high titres, associated with more extensive skin involvement and disease activity
  • anti-nuclear antibodies: 90-95% of patients. usually speckled or centromere pattern. nucleolar pattern, less common, is more specific for Ssc
  • anti-RNA polymerase I and III: specific for dSSc
  • antibodies to U1 RNP: assoc Ssc-pulm HTN, and overlap syndrome, MCTD
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57
Q

mx of scleroderma?

A

MDT approach
management aimed at symptomatic relief, and maximising function

non pharmacological:
- patient education: avoid cold, keep extremities warm
- PTOT for maximising hand function

Pharmacological measures are organ specific
- immunosuppressants: steroids, MTX, Aza, cyclophosphamide
- anti-fibrotic agents
- for complications of ILD, pHTN, Raynauds, GI symptoms, HTN

Surgical

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58
Q

Pharmacological measures for scleroderma?

A

disease specific:
immunosuppression: steroids, MTX, azathioprine, cyclophosphamide, MMF
anti-fibrotic agents: penicillamine, interferon

ILD: steroids and steroid sparing agents
pHTN: prostaglandins e.g epoprostenol, sildenafil (phosphodiesterase inhibitors)
Raynauds: high dose CCB (Nifedipine), pentoxyphylline, prostacyclin, bosentan (endothelin receptor antagonist)

GI symptoms: promotility agents, PPIs
HTN: ACEi (esp during HTN crisis)
Myositis: steroids, MTX, aza
arthralgia, NSAIDs

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59
Q

surgical management in scleroderma?

A

digital sympathectomy for severe raynaud’s phenomenon

amputation for gangrene

correct fixed flexion deformities

drain calcinosis deposits

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60
Q

what are the criteria for scleroderma?

A

ACR 1980 criteria for classification of systemic sclerosis: requires 1 major or 2 minor
- does not include CREST-type patients
- intended for classification, not for diagnosis

major:
- sclerosis of skin proximal to MCPJ/ MTPJ

minor:
- sclerodactyly (skin changes limited to fingers, toes)
- digital tip pitting/ pulp atrophy
- bibasal pulmonary fibrosis

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61
Q

what are the phases of skin changes in scleroderma?

A
  • oedematous phase
  • dermal phase (induration)
  • atrophic phase with contractures
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62
Q

associations and complications of systemic sclerosis?
MSK and cutaneous

A

digital infarcts/ gangrene
flexion contractures
sicca syndrome

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63
Q

associations and complications of systemic sclerosis?
cardio, resp

A

ILD and cor pulmonale
primary pulmonary hypertension
infiltrative CMP/ myocardial fibrosis

reflux pneumonitis, pleural effusion, alveolar cell carcinoma
pericarditis/ pericardial effusion

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64
Q

associations and complications of systemic sclerosis?
renal

A

scleroderma renal crisis
-> tx ACEi

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65
Q

associations and complications of systemic sclerosis?
GI

A

risk of barrett metaplasia 2’ oesophagitis
candida oesophagitis
oesophageal strictures
gastroparesis, oesophageal dysmotility
malabsorption w steatorrhoea (from dilated D2-> bacterial overgrowth)
malnutrition
primary biliary cirrhosis

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66
Q

associations and complications of systemic sclerosis?
neuro

A

entrapment neuropathies- carpal tunnel syndrome, trigeminal neuralgias

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67
Q

associations and complications of systemic sclerosis?
haem

A

anaemia: anaemia of chronic disease, IDA from oesophagitis, B12/folate deficiency from malabsorption, MAHA, aplasia from medications such as MTX

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68
Q

associations and complications of systemic sclerosis?
related to fertility/ pregnancy

A

high risk pregnancies- higher risk fo pregnancy loss and complications, but not an absolute contraindication to pregnancy

erectile dysfunction

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69
Q

what is mixed connective tissue disease?

A

2 or more - SLE, polymyositis, dermatomyositis, SSc
assoc with anti-ribonuclear protein (Anti-RNP) antibody - speckled pattern

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70
Q

prognosis of scleroderma?

A

male (poor)
renal or lung involvement (poor)

skin involvement only: 70% 10 yr survival
renal or lung involvement: 20% 10 yr survival

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71
Q

what antibody is associated with mixed connective tissue disease?

A

anti-RNP antibody, speckled pattern

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72
Q

what is scleroderma renal crisis?

A

presents with accelerated HTN, oliguria, headache, SOB, oedema

may result in renal failure, stroke, retinopathy, MAHA

UFEME: microscopic haematuria, proteinuria but otherwise bland, no cellular casts usually

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73
Q

prevention of scleroderma renal crisis

A

control HTN with ACEi
avoid high dose steroids

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74
Q

treatment of scleroderma renal crisis

A

ACEi

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75
Q

history taking in rheumatoid arthritis?

A

joint pains: distribution, deformity
early morning stiffness: duration, improvement with exercise

AI screen:
rashes
alopecia
sicca symptoms
oral / genital ulcers

disease cx:
- SOB (pulmonary fibrosis)
- eye symptoms
- focal weakness or numbness
- neck stiffness

treatment complications

past medical history
drugs, tcm use
etoh, smoking
family history of AI disease, thyroid disease
job, hobbies, functional impairment and impact on ADLs

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76
Q

history taking in rheumatoid arthritis?
treatment complications

A
  • cushingoid, hirsutism, cataracts
  • hx DM HTN HLD
  • osteoporosis, AVN fem head, mood disturbances
  • anaemia- SOBOE, postural giddiness, GI bleed
  • hx CKD, transaminitis
  • maculopathy
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77
Q

examination of rheumatoid arthritis
- hands and wrists

A

put on pillow and inspect
- joint deformities and arthropathy
- wasting
- dropped fingers 2’ tendon rupture
- rheumatoid nodules over elbow
- psoriatic nail changes

feel for joint warmth and tenderness
palpate hand joints (wrist -> DIPJ)

double punch skin test for thinning skin
tinels sign or surgical scar for CTS

function: gross and fine hand function

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78
Q

examination in rheumatoid arthritis
- general appearance/ cutaneous signs

A

general appearance:
cushingoid
hirsutism
striae
xanthelasma
DM dermopathy
features of CKD
features of SLE- malar rash

inspect hairline and ears for psoriasis

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79
Q

examination in rheumatoid arthritis
- face: eyes, mouth

A

episcleritis, scleritis
cataracts
anaemia
jaundice (haemolytic anaemia, transaminitis)

oral thrush

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80
Q

examination in rheumatoid arthritis
heart lungs neuro

A

heart: AR, MR, pericarditis, pHTN
Lungs; Basal pulmonary fibrosis, effusions

+/- Neuro: ask for any localised weakness. screen EOM, gross sensation, pronator drift, prox myopathy, median nerve palsy, gait

offer abdo exam: splenomegaly for felty’s syndrome, striae

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81
Q

description of rheumatoid arthritis hands

A

bilateral symmetrical deforming polyarthropathy of the hands
- predominantly affecting the PIPJ/MCPJ
- swan neck, boutonniere’s, z thumb deformities
- ulnar deviation
- subluxation at the MCPJ and dorsal subluxation of the ulna at the carpal joint

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82
Q

presentation of rheumatoid arthritis, what to mention

A

diagnosis of RA, due to signs seen

whether disease is active/ quiescent

complications of RA

treatment complications

important negatives: e.g. psoriasis, SLE

function

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83
Q

what would suggest if RA is active/ quiescent

A

if active disease: with warmth, swelling and tenderness over __ joints

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84
Q

other than classic hand description of rheumatoid arthritis, what other things could be mentioned

A

rheumatoid nodules over elbows, extensor surfaces
pyoderma gangrenosum
vasculitis lesions- nail fold infarcts
wasting of intrinsic muscles of hands (disuse)

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85
Q

complications of rheumatoid arthritis: eyes

A

keratoconjunctivitis sicca
episcleritis, scleritis, scleromalacia perforans
blurring of vision 2’ hydroxychloroquine induced maculopathy

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86
Q

complications of rheumatoid arthritis: CVM

A

valvular incompetence - AR, MR
pericarditis

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87
Q

complications of rheumatoid arthritis: respi

A

pulmonary fibrosis
pulmonary hypertension
pleural effusions

Bronchiolitis obliterans with organizing pneumonia
Caplan’s syndrome -combination of RA and pneumoconiosis -> massive lung fibrosis + pulmonary nodules (v rare)

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88
Q

pneumoconiosis + rheumatoid arthritis?

A

caplans’ syndrome

characteristic pattern of fibrosis
5-50 mm well-defined nodules in the upper lung lobes/lung periphery

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89
Q

complications of rheumatoid arthritis: msk

A

atlanto-axial subluxation +/- cervical myelopathy
dropped fingers 2’ tendon rupture

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90
Q

complications of rheumatoid arthritis: abdomen

A

Felty’s syndrome - splenomegaly, neutropenia and RA

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91
Q

complications of rheumatoid arthritis: neuro

A
  • nerve entrapment: carpal tunnel syndrome
  • EOM affected - mononeuritis multiplex or myasthenia 2’ penicillamine or autoimmune mediated
  • mononeuritis multiplex
  • peripheral neuropathy
  • muscle atrophy, proximal myopathy 2’ steroids, penicillamine
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92
Q

Feltys syndrome - features

A

splenomegaly + RA + neutropenia
- assoc anaemia/ pancytopenia
- a/s positive ANA, LNpathy, LOW, vasculitic rash, recurrent infections

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93
Q

Treatment of Felty syndrome

A

DMARDs
pulsed IV steroids
+/- cyclophosphamide

splenectomy in patients who fail medical therapy

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94
Q

how to test hand function

A

coarse motor function: turning of door knob, combing hair
fine function: buttons, opening bottle caps, writing

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95
Q

important negatives to mention in someone with suspected rheumatoid arthritis

A

psoriasis: skin lesions, nail changes
SLE: malar/ photosensitive rash

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96
Q

treatment complications in rheumatoid arthritis

A
  • chronic steroid use
  • anaemia 2’ RA, AIHA, drug induced myelosuppression etc
  • chronic renal failure (may be 2’ chronic NSAID use)
  • transaminitis: 2’ MTX, leflunomide
  • penicillamine use: myasthenia (fatiguable weakness and complex ophthalmoplegia)
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97
Q

different patterns of onset seen in rheumatoid arthritis?

A
  • palindromic: episodic with complete resolution between attacks. usually monoarticular
  • systemic: systemic/ extra articular features
  • polymyalgic: symptoms similar to polymyalgia rheumatica
  • chronic persistent: typical form. relapsing/remitting course over years
  • persistent monoarthritic: usu 1 knee, shoulder jt or hip involvement
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98
Q

causes of anaemia in rheumatoid arthritis?

A
  • Iron deficiency: GI bleed from NSAIDs/ steroid
  • megaloblastic anaemia: pernicious anaemia
  • anaemia of chronic disease
  • hypersplenism from felty’s syndrome
  • aplasia 2’ gold/ penicillamine
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99
Q

poor prognostic indicators for rheumatoid arthritis?

A
  • insidious onset and high activity at onset
  • persistent activity after 1 year - active arthritis, ESR
  • rheumatoid nodules or early erosions within 1 year
  • extra-articular features
  • high levels of RF and anti-CCP ab
  • HLA DR4
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100
Q

Diagnostic criteria for rheumatoid arthritis?

A

american college of rheumatology diagnostic criteria: 4 or more of the following:

  • morning stiffness >1h for >6/52
  • symmetrical arthritis > 6/52
  • arthritis of hand joints >6/52
  • arthritis of >/=3 joints for > 6/52
  • subcutaneous nodules
  • RF positive
  • characteristic XR findings: erosions, periarticular osteopenia
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101
Q

ix of rheumatoid arthritis?

A

diagnostic
- Rheumatoid factor (sensitivity 70-90%, specificity 95%)
- anti-CCP antibodies (more specific), assoc w more severe disease

to assess severity/ disease activity
- ESR, CRP
- XR joints: erosions and periarticular osteopenia

to rule out other complications/ assoc conditions:
- ANA to screen for lupus
- LFTs: many drugs cause derangement of LFTs
- Synovial fluid examination TRO septic or crystal arthropathies

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102
Q

management of rheumatoid arthritis?

A

MDT approach:
nurse clinician- education, counselling
PTOT to maximise function and independence

pharmacological therapies:
depends on severity
analgesia (NSAIDs, paracetamol)
Steroids, DMARDs

surgical therapies

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103
Q

what medical therapies are available in rheumatoid arthritis?

A

steroids

DMARDs: start early to slow disease progression
- sulfasalazine: 1st line in mild disease w/o radiological cx
- HCQ: alternative for mild disease w/o radiological cx
- MTX: first line in mod/severe disease with radiological cx e.g. erosions
- azathioprine: 2nd line (can add to MTX if inadequate control)
- leflunomide
- penicillamine
- gold
- ciclosporin
- infliximab (anti-TNFa)
- etanercept (anti-TNFa)
- anakinra (IL-1R blocker)
- rituximab (anti-CD20)

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104
Q

what are the first line DMARDs for mild rheumatoid arthritis without evidence of radiological complications?

A

sulfasalazine
hydroxychloroquine

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105
Q

side effects of hydroxychloroquine?

A

corneal deposits
Bulls eye retinopathy
reduced peripheral vision

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106
Q

first line in mod/severe rheumatoid arthritis with radiological complications such as erosions?

A

methotrexate

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107
Q

side effects of methotrexate?

A

myelosuppression
hepatotoxic + hepatic fibrosis
pulmonary fibrosis
B12, folate deficiency

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108
Q

side effects of leflunomide?

A

myelosuppression
hepatotoxicity
pulmonary fibrosis

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109
Q

side effects of penicillamine

A

pancytopenia
nephritis

110
Q

side effects of gold

A

marrow suppression, nephropathy

111
Q

indication to start Infliximab (anti-TNFa) therapy in rheumatoid arthritis?

A

inadequate response to at least 2 DMARDs, including MTX

112
Q

side effects of infliximab/ adalimumab: anti TNFa

A

immunosuppression,
reactivation of latent TB
immunosuppression related malignancy

113
Q

side effects of etanercept

A

risk of demyelination

114
Q

side effects of anakinra (IL1- blocker)

A

immunosuppression
injection site reactions
worsening of heart failure
demyelination

115
Q

side effect of rituximab

A

infusion reactions
b cell depletion -> increased risk infections
hep b reactivation

116
Q

treatment options for rheumatoid arthritis in pregnancy?

A

sulfasalazine, hydroxychloroquine, azathioprine

Anti-TNFa not shown to cause increased risk in pregnancy, but currently not recommended

117
Q

surgical options for rheumatoid arthritis?

A

tendon release
Carpal tunnel release
splenectomy in felty’s syndrome

118
Q

what is Z thumb deformity?

A

hyperextension of the first IPJ and fixed flexion and subluxation of the first MCPJ
-> squaring appearance of the hands

119
Q

what is boutonniere’s deformity?

A

hyperflexion of the PIPJ and hyperextension of the DIPJ

  • due to rupture of the central slip of the extensor tendon over the PIPJ with imbalance of flexion and extension forces of the finger
120
Q

what is swan neck deformity of the fingers?

A

hyperextension of the PIPJ and hyperflexion of the DIPJ

due to synovitis of the flexor tendons leading to flexion of the MCPJ w constant effort to extend the finger -> stretching of the collateral ligaments and volar plate of PIPJ -> intrinsic muscle balance leads to swan neck deformity

121
Q

DDx for deforming polyarthropathy of the hands?

A

rheumatoid arthritis
RA type psoriatic arthritis
jaccoud’s arthropathy/ SLE deforming arthropathy (ulna deviation with subluxation of 2-5 fingers at MCPJ- voluntarily correctable)
gouty arthropathy - gouty tophi
osteoarthritis

122
Q

History taking in psoriatic arthropathy?

A

joint pain- distribution, deformity
early morning stiffness - duration, improvement with exercise

AI screen:
rashes
alopecia
sicca symptoms
oral/ genital ulcers

precipitating factors: drugs (BB, ACEi), ETOH

disease complications: SOB (pulmonary fibrosis), eye symptoms, focal weakness, numbness, neck stiffness

treatment complications

past med history
meds: + TCM, OTC
smoking, etoh
family history: AI disease
job hobbies, functional impairment and impact on ADLs

123
Q

examination in psoriatic arthropathy

A

general inspection:
inspect for psoriatic plaques over hairline, ears, neck
cushingoid?
features of SLE- malar rash
gouty tophi

hands and wrists: put on pillow to inspect
- joint deformities
- wasting
- psoriatic nail changes
- feel for joint warmth, palpate joints wrist -> DIPJ
- double pinch skin test for thinning skin

eyes: anaemia, jaundice (haemolytic anaemia, transaminitis)

mouth: thrush

heart: AR, MR, pHTN
lungs: pul fibrosis

offer abdo: plaques
offer to examine for other sites for psoriatic plaques: natal cleft, submammary, umbilicus, inguinal folds

function: gross and fine hand function

124
Q

types of psoriatic arthropathy?

A
  1. mono/oligoarticular (75%)
  2. RA type (symmetrical joint involvement but seronegative, 15%)
  3. OA type (symmetrical terminal joint involvement, 5%)
  4. arthritis mutilans type (bilateral deforming arthropathy, telescoping of the digits)
  5. AS type (sacroiliitis and axial involvement, but the syndesmophytes arise from the lateral and anterior surface and not at the margins unlike AS)
125
Q

other features of psoriatic arthropathy apart from joint and skin changes?

A

dactylitis
tenosynovitis
wasting of intrinsic hand muscles
nail changes - pitting, onycholysis, subungual hyperkeratosis, discoloration of nails
keloid formation over surgical scars

126
Q

any joint disease, what to mention during presentation?

A

whether disease is active or quiescent

impact on function

127
Q

treatment complications to look out for in psoriatic arthropathy?

A

cushings syndrome from chronic steroid use

anaemia (2’ AIHA, drug induced myelosuppression, GI bleed)

pulmonary fibrosis 2’ mtx (psoriasis itself not assoc w ILD)

chronic renal failure possibly 2’ chronic NSAID use

transminitis - 2’ mtx, leflunomide

128
Q

psoriatic arthropathy, how to complete examination

A
  • examine for other joint involvement
  • full skin examination: esp scalp, knees, natal cleft, inguinal/ submammary folds, koebners phenomenon
  • enquire for presence of any aggravating factors
129
Q

what are the types of skin lesions seen in psoriasis?

A
  • plaque
  • guttate (assoc hx of strep infection)
  • pustular
  • erythrodermic
  • inverse psoriasis (intertriginous area without typical silvery scales due to moisture and maceration)
130
Q

typical sites of distribution for psoriatic plaques?

A

extensor surfaces of knees, elbows
scalp
umbilicus
natal clef
intragluteal, submammary folds

131
Q

how to assess severity of psoriatic arthropathy?

A

Psoriasis Area and Severity Index (PASI)

  • assess severity based on area, thickness, redness and scaling of lesions
  • total score 72: scores of < 10, 10-50, >50 for mild, mod, severe respectively
132
Q

what are the unique characteristics of psoriatic lesions?

A

salmon pink hue with silvery scales
koeber’s phenomenon
auspitz’s sign - capillary bleeding when silver scales are picked from plaque
moist red surface on removing of scales (bulkeley’s membrane)

133
Q

what is koebner’s phenomenon, and what other conditions is it seen?

A

new skin lesions at site of cutaneous trauma

assoc:
- psoriasis
- eczema
- vitiligo
- lichen planus
- lichen sclerosus et atrophicus
- molluscum contagiosum

134
Q

ddx for onycholysis?

A

psoriasis
fungal infection
thyrotoxicosis
lichen planus

135
Q

aggravating factors for psoriatic arthropathy

A

emotional stress
alcohol
drugs - acei, BB, lithium, antimalarials
injury to skin
strep infection (assc guttate psoriasis)

136
Q

radiological features of psoriatic arthritis?

A
  • pencil in cup appearance
  • periostitis - “fluffy”
  • destruction of small joints
  • non marginal syndesmophytes in AS type
137
Q

prognosis of psoriatic arthropathy

A

10-15% of patients with psoriasis have psoriatic arthropathy
deforming and erosive in 40%

138
Q

what other joint pathology can patients have especially if psoriatic disease is active?

A

gout - because of hyperproliferation

139
Q

management of psoriatic arthropathy?

A

education
avoidance of aggravating factors

topicals +/- phototherapy (NBUVB, PUVA) for psoriasis

systemic medications

140
Q

topicals for psoriasis?

A

moisturizers
salicylic acid (keratolytics)

coal tar
topical steroids
topical vit D analogue calcipotriol
topical retinoids

141
Q

systemic therapies for psoriatic arthropathy?

A

methotrexate
azathioprine
MMF
acitretin
tacrolimus, cyclosporine

avoid systemic steroids due to risk of psoriasis flare on steroid withdrawal

biologics: infliximab, etanercept

142
Q

indications for systemic therapy in psoriasis?

A
  • failure of topical therapy
  • repeated admissions for topical therapy
  • extensive plaque psoriasis in elderly
  • severe psoriatic arthropathy
  • generalised pustular or erythrodermic psoriasis
143
Q

what is bazex syndrome

A

rare, acral psoriasiform dermatosis associated with internal malignancies, most frequently squamous cell carcinoma of the upper aerodigestive tract.

-> psoriatic lesions on fingers, toes, nose, ears
-> exclude SCC of oropharynx, tracheobronchial tree, oesophagus

144
Q

history taking in ankylosing spondylitis?

A

history of joint pain, early morning stiffness

AI screen
GI symptoms- IBD
psoriasis

disease complications
treatment complications

pmh, drug hx, smoking/etoh
family history
job hobbies, impact on ADL

145
Q

ddx of ankylosing spondylitis?

A

psoriatic arthropathy axial variant
IBD; enteropathic arthritis

146
Q

articular features of ankylosing spondylitis?

A

sacroiliitis
spondylitis
spinal ankylosis
intervertebral discitis
erosion of apophyseal joints

147
Q

extra articular features of ankylosing spondylitis?

A

7As

anterior uveitis
aortic regurgitation
aortitis
AV block
apical lung fibrosis
amyloidosis
achilles tendinitis

148
Q

examination of ankylosing spondylitis?

A

general appearance:
stooped posture, ? posture
loss of lumbar lordosis, marked cervical flexion
abdo breathing to compensate for reduced chest excursion
- look for psoriatic plaques (differential)

trunk:
- stand pt against wall (heel/ buttock against wall): measure wall to occiput distance (should be 0)
- stand away from wall, test for trunk flexion, extension, lateral flexion
- neck ROM
- Schober’s test
- look for psoriatic patch at natal cleft
- chest wall expansion

gait: walk patient to test gait
heel: achilles tendonitis (get patient to stand on toes)

upper limbs: nails for psoriatic pitting, psoriatic patches

then complete examination by examining:
- eyes
- cardiovascular system
- lungs
- assessing fine and gross motor function

149
Q

schober’s test?

A

mark midline between PSIS
mark 5cm below and 10cm above

during forward flexion, distance between should extend by >5cm

150
Q

eye examination in ankylosing spondylitis?

A

anterior uveitis, iritis
- painful, injected eyes, photophobia, sluggish pupillary reflexes

151
Q

cardio/resp exam in ankylosing spondylitis?

A

aortic regurgitation
pulmonary hypertension from restrictive lung disease
apical lung fibrosis

152
Q

abdo exam in ankylosing spondylitis?

A

scars that may indicate previous bowel surgery for IBD (differential)
psoriatic plaques (differential)
hepatosplenomegaly from amyloidosis

153
Q

features of ankylosing spondylitis?

A
  • stooped “question mark” posture
  • fixed flexion of neck with increased wall to occiput distance
  • restriction of movement of lumbar and cervical spine
  • positive schobers test (loss of lumbar spine excursion)
  • restriction of chest wall movements
154
Q

what may suggest chronicity of disease on examination in ankylosing spondylitis?

A

extensive ankylosis involving cervical, thoracic, lumbar spine

155
Q

ix of ankylosing spondylitis?

A

radiography:
- XR pelvis: sclerosis of sacroiliac joint
- xr spine: bamboo spine, squaring of vertebrae, dagger sign (ossification of posterior longitudinal ligament), loss of lumbar lordosis
- CXR for apical fibrosis

bloods:
FBC RP
ESR, CRP
HLA B27 (87% association)

ECG for cardiac conduction abnormalities, pHTN features
2DE: to exclude AR, measure aortic root diameter and pulmonary pressures
Lung function tests: restrictive defect due to pulmonary fibrosis, kyphosis and ankylosis of costovertebral joints

156
Q

what is ankylosing spondylitis?

A

seronegative spondyloarthropathy
- chronic inflammatory arthritis affecting the SI joints with fusion of the spinal vertebrae
- assoc HLA B27

usually affects males, 3-4th decade

157
Q

symptoms in ankylosing spondylitis?

A

back pain worse in the morning and with rest,
improves with activity

158
Q

first signs of ankylosing spondylitis?

A

limited lateral flexion of the lumbar spine is first sign of spinal involvement,
followed by loss of lumbar lordosis

159
Q

what is the heels-hips-occiput test for Ank Spond?

A

ask pt to place heels, hips and occiput against the wall

inability of the occiput to touch the wall

-> measure wall-occiput distance

160
Q

why is there a protuberant abdomen in ank spond

A

restricted chest expansion from fixed spine -> predominantly diaphragmatic breathing
-> protuberant abdomen

161
Q

causes of breathlessness in ank spond?

A
  • restrictive lung disease
  • interstitial lung disease
  • cor pulmonale
  • heart failure 2’ AR
162
Q

conditions that may cause sacroiliitis?

A
  1. ank spond
  2. psoriatic arthropathy
  3. enteropathic arthritis
  4. reactive arthritis
163
Q

diagnosis of ankylosing spondylitis?

A

Rome or New York criteria
based on
1. radiological features of sacroiliitis
2. symptoms of back pain (lumbar spine, dorsolumbar junction)
3. physical signs of limited spinal range of motion in all 3 planes and chest expansion < 2.5cm

164
Q

imaging findings of ankylosing spondylitis?

A

early - erosions, sclerosis of SI joints
later- syndesmophytes in the margins of the lumbosacral vertebrae
advanced- “bamboo” spine

165
Q

management of ankylosing spondylitis?

A

MDT
aims are to relieve symptoms, maximize function and limit disability

education and counselling
genetic counselling

exercises, PTOT involvement

pharmacological:
NSAIDs
DMARDs

surgical therapies

166
Q

pharmacological therapies in ankylosing spondylitis?

A

NSAIDs- analgesia, anti-inflammatory
DMARDs - limited benefits
> can trial MTX, sulfasalazine for patients with predominant peripheral spondyloarthropathy

Biologics: anti TNFa therapies (etanercept, infliximab), anti CD20 (rituximab)

167
Q

surgical therapies in ank spond?

A

joint replacement
spine straightening

168
Q

indications for starting immunomodulators in ank spond?

A

TNF blocking agents recommended for treatment of active AS after having failed treatment for the patient’s predominant clinical manifestation

169
Q

what indices may be used to assess disease activity in ankylosing spondylitis?

A

BASFI (Bath Ank Spon Functional Index); assess level of functional disability

BASDAI (Bath AS disease activity index): self administered questionnaire assessing severity of symptoms

ASAS (assessment in Ank Spond): composite sum of disease activity

170
Q

features of reactive arthritis?

A

triad of urethritis, arthritis, conjunctivitis

usually after urogenital (chlamydia) Or GI (shigella, cambylobacter, salmonella) infection

cx: cirinate balanitis, keratoderma blenorrhagicum

171
Q

causes of charcot joint?

A

metabolic: DM (commonest), renal
infection: syphilis, leprosy
neuro:
- peripheral neuropathy
- spinal cord injury
- myelomeningoecele
- syringomyelia
- HSMN
- congenital insensitivity to pain

172
Q

examination findings of charcots joint?

A

general appearance:
grossly deformed foot/ankle
loss of concavity of foot arch
neuropathic punched out ulcers
amputation
DM dermopathy
features of peripheral vascular disease
ankle foot orthoses

palpate for warmth, swelling, tenderness, thickened nerves, pedal pulses

passive ROM of joints for:
crepitations
instability, hypermobility of the joint

active ROM

sensation:
- loss of propioception and vibration
- loss of pin prick sensation

173
Q

charcot joint:
how to complete examination

A
  • pupils for argyll robertson pupils (syphilis), bilateral ptosis (leprosy)
  • rombergs gait (sensory ataxia with peripheral neuropathy)
  • gait for sensory ataxic gait (high stepping gait, foot shuffling gait)
  • examine back for spinal surgery scar, meningocele
  • neuro exam of UL for dissociated sensory loss (syringomyelia)
  • urine dipstick for glycosuria
174
Q

what is a charcot’s joint?

A

chronic progressive degenerative neuropathic arthropathy resulting from a disturbance from sensory innervation of the affected joint

characterized by joint dislocations, pathologic fractures, debilitating deformities

results in progressive destruction of bone and soft tissues at weight bearing joints

175
Q

aetiology of charcot’s joint?

A

any condition that causes sensory or autonomic neuropathy

complication of diabetes, syphilis, chronic alcoholism, leprosy, meningomyeloele, spinal cord injury, syringomyelia, renal dialsysi, congenital insensitivity to pain

diabetes is most common cause

176
Q

presentation of charcot’s joint?

A

acute inflammation: unilateral swelling, hot, red, effusion, insensate foot -> acute charcot arthropathy

pain can occur

instability and loss of joint function: passive movement of joint may reveal instability

neuropathic ulcers: 40% have concomitant ulceration which complicates the diagnosis and raises concerns that osteomyelitis is present

177
Q

stages of charcot’s foot?

A

atrophic form - usually forefoot with osteolysis with distal metatarsal, X ray shows MT resembling a pencil point

hypertrophic form (mid or rear foot and ankle):
- stage 0: clinical stage with sign and symptoms but no joint deformity
- stage 1: acute (developmental or fragmentation stage)
> periarticular fracture with joint dislocation and unstable deformed foot
> tender, red, swollen mimicking infection
- stage 2: subacute (coalescence stage)
> resorption of bone debris
- stage 3: chronic (reparative stage)
> restabilization
> enlarged and deformed, non tender fusion of the involved fragments

178
Q

ix of charcot’s joint?

A

to confirm diagnosis, assess severity of joint destruction, rule out complications of osteomyelitis
assess underlying aetiology

imaging:
- XR: helps to stage disease, determine if active disease present or if joint is stable
- bone scan / MRI: may help to distinguish between osteomyelitis and charcot arthropathy
- doppler US to rule out DVT

labs: FBC ESR CRP

workup for underlying aetiology

diagnostic procedures
- synovial biopsy
- joint aspiration to rule out septic joint

179
Q

x-ray features of charcot joint

A

osteopenia
periarticular fragmentation of bone
subluxations/ dislocations
fractures
generalised destruction

180
Q

workup for underlying aetiology of charcot joint?

A

RP - renal failure
HbA1c - DM
RPR/VDRL - syphilis -> then LP
B12/folate - peripheral neuropathy
LFT, coags - alcoholism
ALP Ca Phosphate PTH - TRO paget’s disease, hypercalcaemia 2’ metastatic bone lesion

181
Q

some diagnostic procedures for charcot joint

A

synovial biopsy
- small fragments of bone and cartilage debris are embedded in the synovium because of joint destruction
- highly suggestive of charcot arthropathy

joint aspiration to rule out septic joint

demonstrate loss of protective sensation of foot
- semmes weinstein monofilament: positive if 4/10 sites affected

182
Q

treatment of charcot joint

A

medical therapy of acute phase:
immobilization with cast/ brace/ ankle foot orthoses and reduction of stress (partial weight bearing, use crutches)

medical management of post acute phase:
education and professional foot care
protection of involved extremity: custom footwear, brace

surgical options

183
Q

surgical management of charcot joint?

A

osteotomy, arthrodesis, screw and plate fixation, reconstructive surgery, amputation

surgery indicated for malaligned, unstable, nonreducible fractures or dislocations

goal of reconstruction -> create a stable plantigrade foot that can support ambulation

contraindication: active inflammation

184
Q

history taking for dermatomyositis?

A

rash- distribution, photosensitivity
weakness, myalgia- pattern
alopecia
digital gangrene (vasculitis)
raynauds

disease cx:
fatiguability (anaemia)
LOW, LOA (malignancy)
malignancy screen
SOB from ILD
dysphagia

AI screen

treatment complications

185
Q

management of post acute phase in charcot arthropathy?

A

patient education and professional foot care

lifelong protection of involved extremity:
- brace
- custom footwear

total healing process typically takes 1-2 years

186
Q

management of acute phase in charcot arthropathy?

A

immobilization and reduction of stress
- casting x 3-6 months
- metal braces, ankle foot orthoses
- partial weight bearing with assistive devices eg crutches, walkers

187
Q

examination findings for dermatomyositis?

A

general appearance:
- cachexia (malignancy)
- cushingoid (steroids)

hands:
gottron’s
vasculitis, dilated nailfold capillaries
periungual erythema and telangiectasia
raynauds
mechanics hands
calcinosis
clubbing (malignancy)
features of SLE/SSc/RA (overlap syndrome)

upper limbs:
elbows for rashes
tenderness of muscles
proximal myopathy

face:
heliotrope rash
alopecia
shawl sign
weakness of neck flexion
conjunctival pallor (GI/ myeloproliferative malignancies)
radiotherapy marks (NPC)

respi:
pulmonary fibrosis, pulm HTN, signs of lung ca

abdomen:
striae
scars (GI malignancy)
hepatomegaly (liver mets)

knees for rash

**test function: **
hands- gross and fine motor function
LL: gait, squat to stand

188
Q

dermatomyositis, what to request for to complete your examination?

A

request to screen for underlying malignancy:
- breast exam
- respiratory exam
- abdominal exam: GI primary, liver mets
- hx of dysphagia, reflux symptoms and weakness

189
Q

what is gottrons sign

A

scaly violaceous papules over knuckles with erythema that spares the phalanges

190
Q

what are mechanic’s hands?

A

lateral and palmar aspect of fingers become rough and cracked with irregular dirty horizontal lines/fissuring, erythematous red pulp of fingers

191
Q

dermatomyositis, what important negatives to mention in presentation?

A
  • no features of overlap syndrome: SLE, SSc, RA
  • no features of underlying malignancy
192
Q

V shaped erythematous rash over neck and upper chesk and back. may become post inflammatory hyperpigmentation

A

shawl sign

193
Q
A
194
Q

ix of dermatomyositis?

A

aims would be to confirm diagnosis and exclude underlying malignancy

diagnostic:
- CK: raised and reflects disease activity
- Myositis panel: ANA, Anti-Mi2, anti-Jo1
- EMG: myopathic changes
- muscle biopsy: muscle necrosis and phagocytosis of muscle fibres, with interstitial and perivascular infiltration of inflammatory cells

rule out malignancy

195
Q

what other inx may be indicated to rule out malignancy in dermatomyositis?

A
  • ENT evaluation +/- MRI neck and posterior nasal space to look for NPC
  • endoscopic evaluation of GI tract
  • Mammogram and US pelvis/ gynae exam for females
  • PSA in males
  • CT Neck, TAP
196
Q

what other ix may be indicated for other complications of dermatomyositis?

A

dysphagia: barium swallow. for atonic dilated oesophagus

CXR/ HRCT for ILD

197
Q

Bohan and Peter criteria for diagnosis of dermatomyositis?

A

4 out of 5 criteria:
1) cutaneous features
2) symmetrical proximal muscle weakness
3) elevated muscle enzymes (CK, AST, Aldolase, LDH)
4) EMG showing myopathic pattern, spontaneous fibrillation, short duration polyphasic motor potentials, complex repetitive discharges
5) Muscle biopsy: perivascular and perimysial inflammation and infiltration of mononuclear cells, perifascicular necrosis

198
Q

what muscle enzymes may be elevated in dermatomyositis?

A

CK
Aldolase
AST
LDH

199
Q

classification of bohan for dermatomyositis/ polymyositis?

A

group 1: primary idiopathic polymyositis
group II: primary idiopathic dermatomyositis
group III: dermatomyositis or polymyositis a/w malignancy
group IV: childhood dermatomyositis or polymyositis a/w vasculitis
group V: dermatomyositis or polymyositis a/w collagen vascular disease

200
Q

management of dermatomyositis?

A

education and counselling

general:
skin -avoid sun
muscle- bed rest, PTOT
dysphagia- ST, bed elevation

medical treatment:
- steroids
- IVIG, MTX, azathioprine
- CCB e.g. diltiazem for calcinosis

treat underlying malignancy

201
Q

what is dermatomyositis?

A

an idiopathic inflammatory myopathy with characteristic cutaneous findings

202
Q

types of dermatomyositis?

A

dermatomyositis
polymyositis
amyopathic dermatomyositis (just skin features)

203
Q

what are some disorders associated with myositis?

A

drugs - statin, chloroquine, colchicine
infection: CMV, lyme disease
eosinophilic myositis

204
Q

DDx for dermatomyositis?

A

other inflammatory myopathies e.g. inclusion body myositis
drug induced myopathy
hypothyroidism
HIV infection
MND, SMA
myasthenia gravis

205
Q

features of inclusion body myositis?

A

insidious onset
more prominent distal muscle weakness, with involvement of
- wrist and finger flexors in the upper extremities
- quads and ant tibial muscles in legs

asymmetric esp in beginning

206
Q

ix of inclusion body myositis?

A

MRI: inflammation through muscle (vs along fascial planes in polymyositis)

biopsy: fatty infiltration and muscle atrophy

207
Q

drug induced myopathy causes?

A

statin, fibrate
steroid
antimalarials, chloroquine
colchicine
penicillamine

208
Q

differences between MND and dermatomyositis?

A

MND: UMN + LMN signs
usually normal muscle enzymes
EMG: no myopathic changes

209
Q

differences between myasthenia gravis and dermatomyositis?

A

MG:
fatiguability
EOM affected (rarely in DM)
normal muscle enzymes
EMG: decremental response to tetanic train stimulation
anti-AChR antibodies

210
Q

prognosis of dermatomyositis?

A

depends on
- presence of underlying malignancy
- severity of myopathy
- presence of cardiopulmonary involvement

211
Q

what are the myositis specific antibodies?

A
  • anti-Jo1 (more common in polymyositis than dermatomyositis)
  • anti Mi-2
  • Anti-SRP (signal recognition particle): assoc w severe myopathy and aggressive disease
212
Q

anti-Mi-2 antibodies in dermatomyositis assoc w?

A

relatively acute onset
shawl sign

213
Q

what autoantibodies may be found in polymyositis/dermatomyositis?

A

ANA up to 80%
Myositis specific antibodies in ~30% (Jo1, mi2, SRP)
anti-Ro, La, Sm, RNP - > suggest overlap with other connective tissue disease

214
Q

Anti-Ro antibodies + myositis?

A

suggest overlap of PM/DM with SLE/ sjogrens

215
Q

Anti RNP antibodies + myositis?

A

suggest overlap of DM/PM with mixed connective tissue disease
-> better prognosis, less involvement of internal organs
-> may progress to predominance of one connective tissue disorder over time

216
Q

anti-synthetase syndrome?

A

assoc w anti-synthetase antibodies (such as anti Jo1)

up to 30% of DM/PM patients

  • acute disease onset
  • constitutional symptoms e.g fever
  • raynauds
  • mechanics hands
  • ILD
  • arthritis
217
Q

myositis, arthritis, ILD

A

anti-synthetase syndrome

218
Q

anti-MDA 5 antibody in dermatomyositis assoc w?

A

poor prognosis
assoc w rapidly progressive ILD

219
Q

anti TIF 1 gamma antibodies in dermatomyositis assoc w?

A

predicting cancer association

220
Q

antipolymyositis-scleroderma (PM-Scl) and anti ku antibodies + myositis?

A

seen in polymyositis/ SSc overlap

221
Q

what antibodies in myositis panel are important?

A
  • some are assoc with overlap syndromes
  • some are myositis specific: Jo1, Mi2
  • some are assoc w cancer: TIF1
  • some assoc with rapidly progressive ILD: MDA 5
222
Q

medications for Dermatomyositis?

A
  • pulsed IV steroids for induction then tapering oral regime for maintenance
  • DMARDs are steroid sparing
  • cyclophosphamide, rituximab, IVIG for severe/ resistant cases
223
Q

history taking in gout?

A
  • joint pain history
  • autoimmune screen
  • disease complications:
    urate renal stones - renal colic, haematuria
    crystal nephropathy
    tophi/ joint deformity
  • treatment complications:
    NSAID nephropathy
    cushings/ steroid side effects
  • gout precipitatants:
    diet: alcohol, meat, seafood, nuts
    dehydration
    history of psoriasis, lympho/myeloproliferative disease, haemolytic anaemia, polycythaemia
    drugs: diuretics, aspirin, tacrolimus, cyclosporine

pmhx; ESRF
FHX gout

224
Q

DDx of gouty arthropathy?

A

RA
OA
psoriatic arthropathy

225
Q

examination in gout?

A

general appearance:
overweight, male (or females post menopause)
features of metabolic syndrome: HLD, DM

hands/ elbows:
asymmetrical deforming polyarthropathy
tophi
Dupuytren’s contracture (ETOH)
nails: pitting, onycholysis (Ddx psoriasis)

look for tophi at the elbows, pinna of ears, feet (esp 1st MTPJ), ankles and heel (achilles tendon)

screen for psoriatic plaques (differential)

assess function of hands: fine and gross motor
gait

screen for signs of secondary causes

226
Q

examination in gout:
to screen for signs of secondary causes

A
  • signs of alcoholism: parotidomegaly, dupuytren’s contractures
  • signs of ESRF: perm cath, AVF, PD catheter, sallow appearance
  • signs of metabolic syndrome: xanthelasma, DM dermopathy, obesity
  • eyes for pallor, jaundice, plethora: haemolytic anaemia, polycythaemia
227
Q

examination in gout:
how to complete your examination?

A
  • offer abdominal exam:
    > look for PD catheter/ scars (ESRF)
    > Hepatosplenomegaly + look for lymphadenopathy (lympho or myeloproliferative disease)
    > periumbilical psoriatic plaques (DDx)

assess CVRF: BP, serum glucose
urine dipstick for glycosuria, proteinuria (nephropathy), haematuria (urate stones)

detailed history for drugs, diet, alcohol

228
Q

what drugs are assoc w higher risk of gout?

A

diuretics: hydrochlorothiazide, dehydration
aspirin
immunosuppressant drugs: Ciclosporin, tacrolimus
TB meds: pyrazinamide, ethambutol

229
Q

features of gout?

A

asymmetrical deforming polyarthropathy
gouty tophi- soft/ firm, not attached to the extensor tendons

230
Q

presentation of gout

A

chronic tophaceous gout with complications of … :
- description
- arthritis active/ quiescent
- functional impairment
- underlying aetiology: ESRF, myelo/lymphoproliferative disease, psoriasis, metabolic syndrome, haemolytic anaemia, polycythaemia

231
Q

ix for gout?

A

confirmation of diagnosis:
- joint aspiration to visualise crystals
- uric acid
- XR of joints: erosive arthropathy from tophi with overhanging edges

excluding predisposing and associated conditions:
- FBC: polycythaemia, haemolytic anaemia
- RP: ESRF
- Hx of alcoholism: LFT, coags
- CVRF: Lipids, fasting glucose

232
Q

joint aspiration for gout

A

negatively birefringent needle shaped crystals (monosodium urate crystals)

233
Q

joint aspiration for pseudogout?

A

calcium pyrophosphate dyhydrate (CPPD) crystals
-> positively birefringent rhomboid crystals

234
Q

Mx of gout

A

MDT approach:
dietican ; low purine diet
education - avoid precipitating drugs/ foods/ dehydration (red meat, legumes/ bean products)

Management of acute flares

Prevention

235
Q

mx of acute flares of gout?

A

analgesia - NSAIDs (indomethacin)
colchine (500mcg TDS): dose adjusted in renal failure, may be cx diarrhoea
prednisolone 30mg OM x 5 days

intra-articular steroid injection (triamcinolone) for renal failure

236
Q

prophylactic management of gout?

A

allopurinol (xanthine oxidase inhibitor: inhibits uric acid formation)

uricosuric agents: probenecid agent, sulfinpyrazone

237
Q

side effects of allopurinol?

A
  • rash, diarrhoea, drug fever
  • leukopenia, thrombocytopenia
  • allopurinol hypersensitivity syndrome
238
Q

when to start medications for prophylaxis of gout?

A
  • recurrent attacks
  • chronic tophaceous gout
  • renal failure
  • urate nephropathy/ high uric acid levels
  • predisposing cause e.g. myeloproliferative disease, chemo/radiotx which may induce tumour lysis syndrome
239
Q

what is gout?

A

gout is a disorder of purine metabolism, resulting in hyperuricaemia either from overproduction or undersecretion of uric acid
-> result in deposition of urate crystals in the joints or bursae

typically presenting w acute monoarthritis of first MTPJ with pain swelling and exquisite tenderness which peaks within hours and lasts for days

240
Q

what do tophi indicate?

A

severe, recurrent, chronic gout

241
Q

common areas to look for gouty tophi

A

hands, exensor aspect of forearms, olecranon bursae
helix of the ears
1st MTPJ, toes, achilles tendons, infrapatellar regions

242
Q

clinical manifestations of gout?

A
  • asymptomatic hyperuricaemia
  • acute arthritis
  • chronic recurrent arthritis
  • tophaceous gout
  • uric acid nephrolithiasis
  • uric acid nephropathy
243
Q

triggering factors of gout?

A
  • alcohol ingestion
  • foods: meat, seafood, nuts, alcohol, innards
  • drugs: thiazides, aspirin, cyclosporine, tacrolimus, pyrazinamide, ethambutol
  • dehydration and fasting
  • surgery, trauma
244
Q

causes of gout

A

primary - assoc obesity, DM, HTN, high TG

secondary:
- drugs,
- chronic ETOH
- psoriasis
- chronic renal failure
- polycythaemia, haemolytic anaemia, lymphoproliferative, myeloproliferative

245
Q

what is pseudogout

A

acute arthritis resulting from deposition of calcium pyrophosphate dihydrate crystals in the joints which are rhomboid shaped positively birefringent cystals under polarized light

246
Q

types of crystal arthropathies?

A
  • gout
  • pseudogout
  • calcium hydroxyapatite crystal deposition in large joints such as knees, shoulders, affecting the elderly
247
Q

DDx for chronic tophaceous gout

A

florid tendon xanthomata
> yellow, not chalky
> adherent to tendon and not joint
> does not involve the bursae ie. no olecranon/ pinna lesions
> no active arthritis

248
Q

genetics of hereditary haemorrhagic telangiectasia?

A

autosomal dominant (either chr 9 or 12)
mutation in gene encoding vascular endothelial cells

249
Q

features of hereditary haemorrhagic telangiectasia?

A
  • telangiectasia (cluster of non contractile dilated capillaries and venules):
    > cutaneous perioral, oral, telangiectasia or over fingers/toes/trunk
  • AVM (abnormal direct connections between arteries and veins)
    > CNS: intracranial AVM -> cranial nerve palsies, long tract signs if previous bleed, headaches
    > Respiratory AVM
    > GI
    > Spinal: sacral vertebral
    > cardiac failure (high output) due to AVM
250
Q

diagnostic criteria for hereditary haemorrhagic telangiectasia?

A

shovlin criteria
- recurrent epistaxis
- telangiectasia at site other than nasal mucosa
- auto dom inheritance
- visceral involvement: AVM

251
Q
A
252
Q

mx of hereditary haemorrhagic telangiectasia?

A

organ specific treatment

CNS AVM: clipping, neurosurgical excision

pulmonary AVM: embolotherapy, surgical ligation/ resection

GI AVM: photocoagulation, endoscopic cautery

Epistaxis: cautery, laser ablation

supportive measures;
- blood transfusions
- iron supplementation
- for epistaxis: antifibrinolytic agent (Aminocaproic acid)

hormonal therapy with oestrogen

253
Q

respiratory complications in hereditary haemorrhagic telangiectasia?

A

respiratory AVM
->
exertional SOB, orthodeoxia (more breathless on standing due to increased flow through AVM when standing than supine)
cyanosis, clubbing

254
Q

GI complications in hereditary haemorrhagic telangiectasia?

A

hepatic AVM: bruit over liver

recurrent GI bleeding: angiodysplasia, mucosal telangiectasia

255
Q

CNS complications in hereditary haemorrhagic telangiectasia?

A

intracranial AVM-> cranial nerve palsies, long tract signs if prev bleed, headaches

cerebral abscess or embolic stroke due to pulmonary AVM

256
Q

examination of hereditary haemorrhagic telangiectasia?

A

mouth:
- telangiectasia around lips
- inside buccal cavity and on tongue

nose:
- crusted blood (epistaxis)
- telangiectasia around nostrils
- facial telangiectasia

face and eyes:
- conjunctival pallor (epistaxis and GI bleeding)
- look for eye deviation or loss of nasolabial fold to suggest VII palsy secondary to prev stroke/ AVM

lung: auscultate for basal cruit

hand:
- digital and nail bed telangiectasia
- clubbing and cyanosis from pulmonary AVM
- check pronator drift (previous stroke: haemorrhagic from cerebral AVM or ischaemic stroke from pulmonary AVM with embolisation)
- look out for jaundice and stigmata of CLD as differential for telangiectasia

abdo: hepatic bruit from AVM

lower limbs: pitting oedema from heart failure due to left to right shunting

257
Q

Anti MDA5 dermatomyositis?

A

Amyopathic usually
Rapidly progressive ILD
Severe skin manifestations
Worse prognosis

258
Q

Antiphospholipid syndrome diagnostic criteria?

A

At least 1 clinical and 1 lab criteria

clinical
- thrombosis: imaging or histological evidence of thrombosis without inflammation in tissue or organ
- pregnancy morbidity:
1) otherwise unexplained death at >=10 wks of normal fetus
2) or >=1 premature birth <= 34 wks due to eclampsia/ peeeclampsia/ placental insufficiency
3) or >= 3 embryonic (<10 wks) unexplained pregnancy losses

laboratory
APL on >=2 occasions > 12 wks apart, not >5 years prior to clinical manifestation:
- anti cardiolipin
- anti b2 glycoprotein IgM or IgG
- lupus anticoagulant

259
Q

2019 EULAR diagnostic criterion for SLE:
Entry criteria

A

ANA positive

260
Q

2019 EULAR diagnostic criteria for SLE?

A

Need at least one clinical criterion and >= 10 points

Clinical domains:
- constitutional: fever (2)
- haematologic: leukopenia (3), thrombocytopenia (4), autoimmune haemolysis (4)
- neuropsychiatric: delirium (2), psychosis (3), seizures (5)
- mucocutaneous: non scarring alopecia (2), oral ulcers (2), subacute cutaneous or discoid lupus (4), acute cutaneous lupus (6)
- serosal: pleural or pericardial effusion (5), acute pericarditis (6)
- MSK: joint involvement (6)
- renal: proteinuria >0.5g/24h (4), renal biopsy class 2 or V lupus nephritis (8), renal biopsy class 3/4 lupus nephritis (10)

Immunological domains:
- antiphospholipid antibodies: anti cardiolipin or anti b2GP1, or LAC (2)
- complements: low C3/c4
- SLE specific antibodies: anti dsDNA OR anti smith (6)

261
Q

Constitutional features in EULAR Diagnostic criteria for SLE

A

Fever (2)

262
Q

Haematologic features in EULAR diagnostic criteria for SLE

A

Clinical domain
- haematologic:

leukopenia (3),
thrombocytopenia (4),
autoimmune haemolysis (4)

263
Q

Neuropsychiatric features in EULAR diagnostic criteria for SLE?

A

Clinical domain:
- neuropsychiatric:

delirium (2),
psychosis (3),
seizures (5)

264
Q

Mucocutaneous features in EULAR criteria for SLE?

A
  • mucocutaneous:
    non scarring alopecia (2),
    oral ulcers (2),
    subacute cutaneous or discoid lupus (4),
    acute cutaneous lupus (6)
265
Q

Serosal features of EULAR Diagnostic Criteria for SLE

A

Pleural or pericardial effusion (5)
Acute pericarditis (6)

266
Q

Musculoskeletal features of EULAR diagnostic criteria for SLE?

A

Joint involvement (6)

267
Q

Renal criteria for EULAR diagnostic criteria for SLE

A

Proteinuria >0.5g/ day (4)
Renal biopsy class 2 or 5 lupus nephritis (8)
Renal biopsy class 3 or 4 lupus nephritis (10)

268
Q

Immunological criteria EULAR diagnostic criteria for SLE?

A

Antiphospholipid antibodies: (2)
Anti cardiolipin or LAC or anti B2GP1

Complements:
Low c3 or c4 (3) , low c3 and c4 (4)

SLE specific antibodies:
Anti dsDNA or anti smith (6)

269
Q

what is Behcet’s disease?

A

auto-inflammatory systemic vasculitis of unknown etiology.

characterized by mucocutaneous manifestations:
- recurrent oral and genital ulcerations
- ocular manifestations, especially chronic relapsing uveitis
- systemic vasculitis involving arteries and veins of all sizes.
- polyarthritis can be seen

270
Q

diagnosis of Behcet’s disease?

A

mouth ulcers that recur at least three times a year, together with two of the following symptoms:

  • Uveitis
  • Typical skin rashes
  • Genital ulcer
  • A positive pathergy test (a special form of skin prick test)
271
Q

management of behcet’s disease?

A

symptomatic, no cure

Smoking cessation

Medications:
Topical steroids for oral and genital ulcers
Colchicine: helps with oral and genital ulcers
Apremilast (PDE4 inhibitor, reduces TNFa): helps with oral ulcers

NSAIDs: for arthritis
Corticosteroids: for severe disease to control inflammation
Biologics

PACES 2024 (14 decks)

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